Carcinoid of uncertain primary site?

[TMZ2007]

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Got a middle aged F with hirsuitism for some time originally taken to OR by GYN about 3 mos ago for excision of slightly enlarged solid/cystic ovary by U/S (I guess they hoped to get a PCOS ovary?). Salpingo-"oophorectomy" showed normal fallopian tube and typical carcinoid tumor, no ovary. Taken back to OR this week with symptomatic small bowel obstruction and multifocal pelvic/peritoneal disease on CT. Path on excised bits shows carcinoid everywhere (intramural small bowel, mesentery soft tissue nodules, multiple lymph nodes, both ovaries and remaining fallopian tube, etc). Appendix reportedly negative in OR, not submitted. No liver or pancreatic or lung masses by CT. No carcinoid syndrome or elevated serum hormones.

Any advice or helpful hints on determining a primary site? Anything that would push you one way or another? (Primary ovary to pelvis and peritoneum vs. primary small bowel to pelvis and peritoneum?) Does it matter? Or do you just wuss out and report "Metastatic well-diff neuroendocrine tumor, please correlate with clinical impression for location of primary site" or some such nonsense? Thanks for your opinions.

 

[Enkidu]

Any advice or helpful hints on determining a primary site? Anything that would push you one way or another? (Primary ovary to pelvis and peritoneum vs. primary small bowel to pelvis and peritoneum?) Does it matter? Or do you just wuss out and report "Metastatic well-diff neuroendocrine tumor, please correlate with clinical impression for location of primary site" or some such nonsense? Thanks for your opinions.

You can use clinical correlation to determine the site of a primary tumor? That would be one a hell of a clinician.

 

[mlw03]

Wouldn't it be prudent for this patient to have an appendectomy, which should be entirely submitted for histology? I'd think a small appendiceal carcinoid that's spread would fit with your findings. But I'm not sure it matters from a treatment perspective if the primary site can be identified or not.

 

[deleted314957]

You can use clinical correlation to determine the site of a primary tumor? That would be one a hell of a clinician.

depending on the case it can be quite useful. i recently had a metastatic paragangloima and pheochromocytoma could essentially be excluded by normal adrenal ct.
but to address the op's broader question--it really does not matter much in most such cases. perhaps a syndromic manifestation would be important for family counseling.

 

[pianoguy05]

Wouldn't it be prudent for this patient to have an appendectomy, which should be entirely submitted for histology? I'd think a small appendiceal carcinoid that's spread would fit with your findings. But I'm not sure it matters from a treatment perspective if the primary site can be identified or not.

100% agree c this; it's what I was going to note but you beat me to it. Great minds....

 

[mlw03]

whoa now people. we're actually discussing pathology in this thread. i thought this forum was devoted to the ills of the business side of pathology?

just kidding. nice to see this every now and then. i should know this being so fresh from residency, but i'd think there could be at least some immunos that could make particular sites more or less likely primaries. i also remember being taught that ovarian tumors had certain gross and microscopic characteristics that could help determine if primary or metastatic is more likely.

 

[GlassPusherJack]

Got a middle aged F with hirsuitism for some time originally taken to OR by GYN about 3 mos ago for excision of slightly enlarged solid/cystic ovary by U/S (I guess they hoped to get a PCOS ovary?). Salpingo-"oophorectomy" showed normal fallopian tube and typical carcinoid tumor, no ovary. Taken back to OR this week with symptomatic small bowel obstruction and multifocal pelvic/peritoneal disease on CT. Path on excised bits shows carcinoid everywhere (intramural small bowel, mesentery soft tissue nodules, multiple lymph nodes, both ovaries and remaining fallopian tube, etc). Appendix reportedly negative in OR, not submitted. No liver or pancreatic or lung masses by CT. No carcinoid syndrome or elevated serum hormones.

Any advice or helpful hints on determining a primary site? Anything that would push you one way or another? (Primary ovary to pelvis and peritoneum vs. primary small bowel to pelvis and peritoneum?) Does it matter? Or do you just wuss out and report "Metastatic well-diff neuroendocrine tumor, please correlate with clinical impression for location of primary site" or some such nonsense? Thanks for your opinions.

is there biopsy material left for immunostains? i would start with serotonin for small bowel (my numero uno vote). small bowel carcinoids may not produce carcinoid syndrome w/o liver mets and they may involve secondary sites extensively compared to primary tumor size. and upper scope for a stomach primary. maybe a gastrin stain.

 

[gbwillner]

I'm sure endocrinology was done? What was abnormal? Clinical Hx/labs is probably the best way...

 

[KCShaw]

I suppose one might come up with a likely source based on the balance of probabilities, but at this point I'm not sure one could be definitive. Interesting though. I agree with the suspicion it may have been prudent to take the appendix, though in the grand scheme of things I'm not sure it matters -- although I don't really know if there would be significant prognostic or treatment differences in this kind of case.

 

[TMZ2007]

I know it's weird, I'm not sure why they didn't take the appendix other than the op note stated that it "appeared normal". The surgeon was not specialty surg onc trained as far as I know.

Serum hormone levels are negative and characteristic signs/symptoms for carcinoid syndrome are not present.

Update: it appears the attending slightly wussed out and ended up saying something like "small bowel is favored to be the primary site, with mets to other locations" with the reasoning that small bowel is the more likely primary site, statistically speaking. No further workup was done (other than basic neuroendocrine markers and a Ki-67). No mention of the appendix or recommendation for appendectomy.

 

[KCShaw]

Given the previously described extensive involvement, it may not matter. It's not like finding the head of the snake and cutting it off magically kills everything else. I think hedging on the primary is likely reasonable, given the situation. No-one knows if the site of origin really has anything significant to do with prognosis or treatment, given the mets?